Action Points

Note that this observational study suggested that treatment with corticosteroids was associated with a higher rate of complete response in IgA vasculitis than treatment with colchicine alone was.

Be aware that one would expect sicker patients to receive the stronger immunosuppressant, so the better outcomes seen in this group is more compelling than in the usual observational framework.

For adults with immunoglobulin (Ig)A vasculitis who have organ involvement, treatment with corticosteroids alone may be a reasonable first-line approach, but the utility of adding cyclophosphamide is unclear, according to a report from the French Vasculitis Study Group.

In the study, published in Arthritis & Rheumatology, on three different statistical analyses, patients treated with corticosteroids or corticosteroids plus cyclophosphamide were found to be more likely to have a treatment response than those given colchicine were, according to Benjamin Terrier, MD, of Hôpital Cochin in Paris, and colleagues. The analyses used were as follows:

"Immunoglobulin A vasculitis, formerly called Henoch-Schonlein purpura, is an immune complex small vessel vasculitis with IgA1-dominant immune deposits," the researchers explained. It is the most common systemic vasculitis of childhood, with an annual incidence of 3 to 26 per 100,000, but is far less common among adults, with an incidence of 0.1 to 1.8 per 100,000 each year.

Experience in treating adults has been limited, and management is further complicated by the fact that the disease often follows a benign course and spontaneous remission can occur.

There has been only one prospective, open-label study of treatment in adults to date, which compared steroids alone or with additional cyclophosphamide among 54 patients with severe IgA vasculitis. In that study, there was no difference at 1 year in remission, renal effects, or adverse events, but survival tended to be better in the combination group (96% versus 79%, P=0.08). "Hence, the optimal therapeutic strategy has yet to be defined in adults," Terrier and colleagues wrote.

To more fully examine the spectrum of disease and efficacy of treatment in adults, the researchers conducted a survey in which they identified 260 cases in France diagnosed from 1990 to 2015.

Patients were considered to have IgA vasculitis if they had purpura, histologically confirmed small vessel vasculitis and IgA deposits, and renal, joint, or intestinal involvement.

A complete response to treatment was defined as an overall improvement in all clinical domains, and for patients with renal disease, by proteinuria below 0.5 g/day, clearance of hematuria, and a decline in glomerular filtration rate of no more than 20%.

Partial response was defined as improvement in at least half of the disease manifestations, and for renal disease, an improvement in proteinuria of at least 50% and worsening of glomerular filtration rate by no more than 20%.

The data were analyzed in a multivariable logistic regression to identify factors independently associated with treatment response. To account for indication bias, the researchers also estimated a propensity score with adjustment for age, sex, gastrointestinal bleeding, proteinuria, and necrotic purpura. The team also used inverse probability weighting on the propensity score.

The mean age of the patients was 50, and 63% were men. All had cutaneous purpura, one-third had constitutional symptoms, almost two-thirds had arthritis or arthralgia; gastrointestinal involvement was present in more than half of the patients, and renal involvement was seen in 70%. A total of 30% were in renal failure at baseline.

Treatments included colchicine alone in 27 patients, corticosteroids alone in 122, and the corticosteroid-cyclophosphamide combination in 35. Other medications used in small numbers of patients were dapsone, rituximab (Rituxan), mycophenolate mofetil (Cellcept), and hydroxychloroquine. No specific treatments were given to 66 patients.

Compared with patients with no treatment or who had colchicine only, those given steroids or cyclophosphamide were more often male, to have necrotic purpura, to have renal involvement with higher levels of proteinuria and hematuria, and to more often have severe gastrointestinal symptoms.

Sufficient follow-up data for analysis were available for 127 patients.

Complete or partial responses were seen in 80% of patients receiving corticosteroids, 77% of those also given cyclophosphamide, and 59% of those given colchicine. Among those with no treatment, 85% experienced spontaneous remissions at either 6 or 12 months.

The researchers also compared the efficacy of corticosteroids alone or in conjunction with cyclophosphamide, and found discordant results: On the multivariate logistic regression model and the propensity score adjusted model, there were no differences in the likelihood of achieving a complete or partial response, but with the use of inverse probability weight on the propensity score, corticosteroids plus cyclophosphamide were more effective (OR 1.79, 95% CI 1-3.20, P=0.049).

During a median follow-up of 17.2 months, there were eight deaths, three of which were considered directly related to IgA vasculitis, and eight additional patients developed end-stage renal disease.

Because of the discordant results on the analyses of steroids alone or in combination with cyclophosphamide, no clear conclusions can be drawn, the researchers cautioned. In addition, the small number of patients given colchicine or the cyclophosphamide combination may have limited the power of the analyses.

"However, given the potential adverse events related to the adjunction of cyclophosphamide in this condition, corticosteroids alone appear to be a reasonable first-line approach in patients with systemic IgA vasculitis, except in very severe presentations in which decisions should be made individually," they concluded.

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